中国实验血液学杂志最新文献

Objective: Serological and molecular biology methods were used to identify the blood type of a patient with forward and reverse ABO typing inconsistency, and to explore the genetic characteristics of this blood type.

Methods: The ABO phenotype of the proband was identified by tube method, and the ABO blood group genotype of the proband and her parents was determined by fluorescent PCR. The 7 exons of the ABO gene were directly sequenced and analyzed.

Results: According to preliminary serological identification, the ABO phenotype of this patient was Bel subtype. Genotyping tests showed that the ABO genotype of the proband and her father was B/O1 , and her mother was O1/O1. Sequencing of exons revealed novel heterozygous variations in exon 1: c.16_17delinsTGTTGCA.

Conclusion: The Novel variations in exon 1 led to Bel subtype in the ABO blood group of the proband, and these variations are heritable.

Objective: To explore the key factors affecting plasma clot retraction and optimize the experimental method of plasma clot retraction, in order to study the regulation of platelet function and evaluate the modulatory effects of drugs on plasma clot retraction.

Methods: The effects of different concentrations of thrombin, Ca2 ⁺ and platelets on plasma clot retraction were studied, and the detection system of plasma clot retraction was optimized. The availability of the detection system was then validated by analyzing the regulatory effects of multiple signaling pathway inhibitors on plasma clot retraction.

Results: Through the optimization study of multiple factors, platelet rich plasma (PRP) containing 0.5 mmol/L Ca2 ⁺ and 40×10⁹/L platelets was treated with 0.2 U/ml thrombin to perform plasma clot retraction analysis. After treatment with thrombin for 15 min, plasma clot retracted significantly. After treatment with thrombin for 30 min, the percentage of plasma clot retraction was more than 50%. The regulatory effects of multiple signaling pathway inhibitors on plasma clot retraction were studied in this detection system. PKC inhibitor Go 6983 exhibited a significant inhibitory effect on plasma clot retraction, while PI3K inhibitor Ly294002 and p38 MAPK inhibitor SB203580 slightly suppressed plasma clot retraction.

Conclusion: PRP containing 0.5 mmol/L Ca2 ⁺ and 40×10⁹/L platelets can be induced with 0.2 U/ml thrombin to conduct plasma clot retraction analysis, which can be used to study the regulation of platelet function and evaluate the modulatory effects of drugs on plasma clot retraction.

Objective: To investigate the clinical characteristics and treatment of relapsed CD5⁺ diffuse large B-cell lymphoma (DLBCL).

Methods: The data of a patient with CD5⁺ DLBCL was collected, and its clinical characteristics and treatment outcome were analyzed.

Results: The patient developed hemophagocytic syndrome and achieved complete remission (CR) after 6 cycles of R-ECHOP chemotherapy, then relapsed. After 2 cycles of PD-1 inhibitor combined with lenalidomide treatment, the patient achieved CR again accompanied by a decrease of interleukin (IL)-10 expression level. After a total of 15 cycles of chemotherapy, the patient remained in CR for 24 months, and the level of IL-10 remained in the normal range.

Conclusion: PD-1 inhibitor combined with lenalidomide regimen may be a new treatment for relapsed CD5⁺ DLBCL.

Objective: To explore and analyze the clinical features and prognostic factors of secondary intestinal diffuse large B-cell lymphoma (SI-DLBCL), in order to provide reference for the basic research and clinical diagnosis and treatment of secondary lymphoma of rare sites in the field of hematology.

Methods: The clinical data of 138 patients with SI-DLBCL admitted to Fujian Medical University Union Hospital from June 2011 to June 2022 were collected and sorted, the clinical and pathological features, diagnosis, treatment and prognosis were analyzed. Cox regression risk model was used to conduct univariate and multivariate analysis on the prognostic risk factors.

Results: Among the 138 patients with SI-DLBCL included in this study, 85 (61.59%) were male, 53 (38.41%) were female, the median age of onset was 59.5 (16-84) years, the clinical manifestations lacked specificity, the first-line treatment regimen was mainly chemotherapy (67.39%), 94 cases (68.12%) received chemotherapy alone, 40 cases (28.98%) were treated with chemotherapy combined with surgery, and 4 cases (2.90%) were treated with surgery alone. The median follow-up time was 72 (1-148) months. Among the 138 patients with SI-DLBCL, 79 (57.25%) survived, 34 (24.64%) died, 25 cases (18.12%) lost to follow-up, the PFS rates of 1-year, 3-year and 5-year were 57.97%, 49.28% and 32.61%, and the OS rates of 1-year, 3-year and 5-year were 60.14%, 54.35% and 34.06%, respectively. The results of univariate Cox regression analysis showed that age, Lugano stage and IPI score were the influencing factors of OS in SI-DLBCL patients, and age, Lugano stage and IPI score were the influencing factors of PFS in SI-DLBCL patients. The results of multivariate Cox analysis showed that Lugano stage was an independent prognostic factor affecting OS and PFS in SI-DLBCL patients.

Conclusion: Patients with SI-DLBCL are more common in middle-aged and elderly men, and the early clinical manifestations lack specificity, and the first-line treatment regimen is mainly R-CHOP chemotherapy, and Lugano stage is an independent prognostic factor affecting OS and PFS in SI-DLBCL patients.

Objective: To understand the etiology, clinical characteristics and prognosis of secondary hemophagocytic syndrome (HLH), so as to improve the understanding of HLH and reduce the rates of misdiagnosis and missed diagnosis of HLH.

Methods: A retrospective study was conducted to analyze the cause, clinical characteristics, laboratory findings, therapy and outcomes of 75 adult patients with secondary HLH admitted to our hospital from January 2015 to December 2021. Follow-up continued until the last discharge time.

Results: Among 75 patients, infection-related HLH was the most common (45.33%), followed by lymphoma-related HLH (17.33%). Fever was the most common clinical manifestation (97.67%). Laboratory indicators such as NK cell activity (98.31% low or absent), sCD25 (93.22% increased), and serum ferritin (94.44% elevated) had higher sensitivity in diagnosis. By comparing the clinical manifestations and laboratory indicators of HLH patients with different causes, sex, lymph node enlargement and bone marrow morphology were more valuable for the diagnosis of primary disease (all P <0.05). By comparing the treatment and clinical outcomes of HLH patients with different causes, the highest clinical remission rate (83.3%) was achieved in patients with autoimmune disease-related HLH treated with hormone+cyclosporine (P <0.05). The overall 12-month survival rate of all patients was 26.7%, in which the infection-related HLH was the lowest (14.7%) while autoimmune disease-related HLH was the highest (63.6%).

Conclusion: The causes and clinical characteristics of adult secondary HLH are varied, with poor prognosis and heterogeneity in disease severity. It is important to identify HLH cause early for diagnosis and needed to further understand HLH.

Objective: To explore the relationship between age at diagnosis and clinical outcomes in children with chronic immune thrombocytopenia (cITP).

Methods: A retrospective analysis was conducted on 117 children with cITP, according to the age at which the patient was diagnosed with ITP, they were divided into two groups: the<10 year old group and the ≥10 year old group, the general information and clinical outcomes of the two groups of children were compared and analyzed. Logistic regression analysis was used to analyzed the impact of age at the time of diagnosis on clinical outcomes, and the predictive evaluation value of age on outcomes was assessed by the receiver operation characteristic.

Results: Compared with the group with diagnosed age<10 years old, the proportion of second-line drug treatment (41.46% vs 18.42%) in the diagnosed age group ≥10 years old was significantly higher, and the proportion of ≥grade 3 bleeding (36.59% vs 13.16%) was significantly higher, which was significant statistical differences ( P < 0.05). However, there was no statistically significant difference in the proportion of untreated CR between the two groups after 5 years of diagnosis (P >0.05). Logistic regression results show that age (older) was an unfavorable/dangerous influencing factor for the occurrence of ≥grade 3 bleeding after second-line treatment (OR >1, P < 0.05). For the occurrence of CR after 5 years of diagnosis without treatment, age was not the influencing factor (P >0.05). ROC analysis showed that age have a certain predictive and evaluative effect on the use of second-line treatment and the occurrence of ≥grade 3 bleeding, with AUC of 0.741(95%CI : 0.549-0.938) and 0.786(95%CI : 0.605-0.940), respectively. However, there was basically no predictive evaluation value for the occurrence of CR after 5 years of diagnosis without treatment.

Conclusion: Older age at the time of diagnosis is not conducive to the prognosis of cITP patients.

Objective: To investigate the prognostic value of lymphocyte-to-monocyte ratio (LMR) and CD163⁺tumor-associated macrophages (TAM) in patients with diffuse large B cell lymphoma (DLBCL).

Methods: Peripheral blood and lymph node tissues were collected from 63 newly diagnosed DLBCL patients. LMR was calculated by the number of lymphocytes and monocytes in peripheral blood from the result of blood routine examination. The level of CD163⁺TAM in lymph nodes was detected by immunohistochemistry. The cut-off values of LMR and CD163⁺TAM were determined by ROC curves, and the prognostic value of LMR and CD163⁺TAM in DLBCL patients was analyzed.

Results: The LMR level of 63 newly diagnosed DLBCL patients was 3.69±1.71, and the median value of CD163⁺TAM was 26/HPF. The number of CD163⁺TAM was negatively correlated with LMR (r =-0.58) and positively correlated with monocyte count (r =0.46). The cut-off values of LMR and CD163⁺TAM determined by ROC curve were 2.95 and 29/HPF, respectively, and based on this, the patients were divided into low LMR group and high LMR group, as well as low CD163⁺TAM group and high CD163⁺TAM group. The proportion of patients with clinical stage III-IV, IPI score 3-5 and bone marrow infiltration in the low LMR group were higher than those in the high LMR group (P < 0.05). The proportion of patients with clinical stage III-IV, IPI score 3-5, elevated LDH level and bone marrow infiltration in the high CD163⁺TAM group were higher than those in the low CD163⁺TAM group (P < 0.05). There was a positive correlation between LMR and OS (r =0.43) and a negative correlation between CD163⁺TAM and OS (r =-0.65). DLBCL patients with low LMR and high CD163⁺TAM had shorter OS (P < 0.05).

Conclusion: Low LMR and high CD163⁺TAM can be used as biological markers for poor prognosis of DLBCL patients.

Exportin-1 (XPO1) is a major transporter for hundreds of proteins. Selinexor is the first generation XPO1 inhibitor. At present, selinexor has gained more attention in the application of multiple myeloma (MM). Meanwhile, the latest clinical trials have confirmed that whether it is a single agent or combined with other chemotherapy regimens, selinexor can also achieve good therapeutic effects in patients with leukemia and lymphoma. This review summarizes the results of preclinical studies and clinical trials of selinexor in treatment of non-MM hematological malignancies, aiming to explore how to choose single agent or in combination with other regimens as induction chemotherapy.

With the development of transfusion medicine, platelet pathogen contamination is of increasing concern to the industry. Currently, pathogen reduction technology (PRT) has been successfully applied to platelets and achieved good results. This paper provides an overview of the research progress of commercial platelet PRT, a comprehensive analysis of the current application status of platelet PRT, preclinical mechanism studies, clinical cohort studies and alternative or complementary strategies, and makes recommendations to provide a scientific basis for safeguarding blood safety in China and developing platelet PRT products applicable to our national conditions.

Objective: To investigate which indicator is more advantageous when using arterial oxygen saturation (SaO₂) and fingertip pulse oxygen saturation (SpO₂) for blood oxygen detection in patients with hyperleukocytic acute leukemia (HAL).

Methods: In this prospective research, the difference between SaO₂ and SpO₂ of 18 HAL patients (observation group) and 14 patients (control group), as well as the relationship between the difference and white blood cell (WBC) counts were analyzed.

Results: SaO₂ was lower than SpO₂ in the observation group (P <0.05), and SpO₂-SaO₂ difference was positively correlated with WBC counts (r =0.47). However, there was no statistical difference between SaO₂ and SpO₂ in the control group. SaO₂ and PO₂ showed a downward trend with the prolongation of detection time after arterial blood was collected in the observation group, but there was no statistical difference. There was no downward trend of SaO₂ and PO₂ in the control group.

Conclusion: HAL patients have a phenomenon where SaO₂ is lower than SpO₂, that is pseudohypoxemia, and this phenomenon may be caused by excessive consumption of oxygen by the leukemia cells in vitro SpO₂ can be monitored bedside in real time and is non-invasive, it is a better way to detect the blood oxygen status of HAL patients.